Infant, Secondary AIHA Treatment Challenging, Indian Study Shows

An examination of medical records of Indian children with autoimmune hemolytic anemia (AIHA), a group of disorders that includes cold agglutinin disease, found managing infants and those with secondary AIHA challenging, with almost one-third of patients needing treatment beyond steroids, a study reveals.

The study, “Clinical Profile and Outcome of Childhood Autoimmune Hemolytic Anemia: A Single Center Study,” was published in the journal Indian Pediatrics.

AIHA is a group of disorders caused by antibodies that mistakenly target red blood cells, leading to their destruction. Warm AIHA occurs at body temperature, whereas cold agglutinin disease (CAD) is a form of AIHA triggered by cold temperatures. 

AIHA can be divided into primary, with unknown cause, and secondary, meaning it is caused by other diseases, such as infections, other autoimmune disorders, or malignancies. 

Given the scarcity of data on AIHA in children living in India, a team of investigators at the Sir Ganga Ram Hospital in New Delhi, India, examined the medical records of 50 AIHA children, ages under 18, from a single center in northern India to highlight clinical parameters and treatment outcomes.

AIHA was diagnosed based on a positive direct antiglobulin test (Coombs test) that detected the presence of antibodies attached to the surface of the red blood cells. Additional blood tests confirmed the diagnosis. The children ranged in age from 1.5 months to 17 years. 

When first examined, the most common clinical feature was a pale appearance (pallor) in all patients, followed by fever in 68% and a yellowish appearance (jaundice) in 60%. Enlarged liver was more prominent (90%) than enlarged spleen (38%). 

Overall, patients had very low levels of hemoglobin (the protein within red blood cells that carries oxygen) at presentation, with 72% presenting severe or very severe anemia. However, children who developed AIHA during infancy had more severe anemia than those who developed symptoms later in life.

Admission to the intensive care unit was required for 28% of children. 

The team noted increased numbers of white blood cells in 54% of participants, while a lower white cell count was found in 8%. An increase in immature red blood cells occurred in 74% of cases, whereas decreased levels were seen in 26%. Elevated lactate dehydrogenase (LDH) concentration, a marker of red blood cell destruction, was seen in 86% of children. 

Based on blood tests, cold AIHA was found in 35% of children, warm AIHA occurred in 28%, and 15% of cases had a mix of cold and warm AIHA. Paroxysmal cold hemoglobinuria, a similar AIHA condition caused by infection, was found in 4% of the children.

Overall, researchers identified a cause for AIHA in 36% of children. The most common causes of secondary AIHA were infections and autoimmune diseases, both occurring in 10% of the patients. 

In two-thirds of patients, steroid therapy was the only prescribed treatment; other medicines used in the remaining patients included intravenous (into-the-vein) immunoglobulin, rituximab, cyclosporine, and immunosuppressive medications.

Children with secondary AIHA required significantly more prolonged treatment (11 months) than primary patients (6.6 months), as did those needing multiple medications compared to patients treated with steroids only (13.3 vs. 7.5 months). 

Sustained complete remission was achieved in 58% of cases, and complete remission is 32%. Although relapse was reported in 26% of participants, 61.5% showed good responses to steroids. 

While one patient improved spontaneously without treatment, one child with AIHA secondary to a malignancy died from infection and low platelets. 

“We present Indian data on pediatric AIHA and underscore the preponderance of AIHA in younger children,” the investigators concluded. “Treating AIHA in children can be challenging and may need prolonged and complicated therapy, especially in secondary AIHA.”